Various indicators for the assessment of hospitals' performance status: Differences and similarities

Background: Hospitals are the most costly operational and really important units of health system because they consume about 50-89 of total health resources. Therefore efficient use of resources could help in saving and reallocating the financial and physical resources. Objectives: The aim of this study was to obtain an overview of hospitals' performance status by applying different techniques, to compare similarities and differences between these methods and suggest the most comprehensive and practical method of appraisal for managers and policy makers. Patients and Methods: This is a cross sectional study conducted in all hospitals of Ahvaz (eight hospitals affiliated with Jundishapur University of Medical Sciences and eight non-affiliated hospitals) during 2007 to 2011. Two kinds of data were collected through separate special checklists. Excel 2007 and Windeap 2.1 software were applied for data analysis. Results: The present findings show that the average of bed occupancy rate (BOR) in the studied hospitals was about 65.91 ± 1.16. The maximum number of inefficient hospitals in the present study happened in the years 2007, 2008 and 2010 (four hospitals) but there were two hospitals in the third part of the present graph which had maximum level of efficiency and optimal level of productivity in the years 2007 and 2009. Data Envelopment Analysis (DEA) showed that the mean score of technical efficiency for the studied hospitals is 0.924 ± 0.105 with the minimum of 0.585 ± 0.905 for hospital number 1. Furthermore It shows that only five hospitals (31.25) reach complete technical efficiency (TE) scores across all five years of 2007-11 (TE = 1). Conclusions: Results of the present and similar studies should be considered for the future planning and resource allocation of Iranian public hospitals. At the same time it is very important to consider need assessment results for each region according to its potentials, population under the coverage and other geographical and cultural indices. Furthermore because of potential limitations of each of the above models it is highly recommended to apply different methods of performance evaluation to reach a complete and real status view of the hospitals for future planning. © 2014, Iranian Red Crescent Medical Journal; Published by Kowsar Corp

Comparison of Common Monogenic Defects in a Large Predominantly Antibody Deficiency Cohort

BACKGROUND: Predominantly antibody deficiencies (PADs) are the most common primary immunodeficiencies, characterized by hypogammaglobulinemia and inability to generate effective antibody responses. OBJECTIVE: We intended to report most common monogenic PADs and to investigate how PAD patients who were primarily diagnosed as agammaglobulinemia, hyper IgM syndrome (HIgM) and common variable immunodeficiency (CVID) have different clinical and immunological findings. METHODS: Stepwise next generation sequencing and Sanger sequencing were performed for confirmation of the mutations in the patients clinically diagnosed as agammaglobulinemia, HIgM and CVID. RESULTS: Among 550 registered patients, the predominant genetic defects associated with agammaglobulinemia (48 BTK and 6 μ heavy chain deficiencies), HIgM (21 CD40L and 7 AID deficiencies) and CVID (17 LRBA deficiency and 12 atypical ICF syndromes) were identified. Clinical disease severity was significantly higher in patients with μ heavy chain and CD40L compared to patients with BTK (P = 0.003) and AICDA (P = 0.009) mutations. Paralysis following live polio vaccination was considerably higher in patients with μ heavy chain deficiency compared with BTK deficiency (P <0.001). We found a genotype-phenotype correlation among patients with BTK mutations regarding clinical manifestation of meningitis and chronic diarrhea. Surprisingly, we noticed that first presentations in the majority of ICF patients were respiratory complications (P = 0.008), while first presentations in LRBA patients were non-respiratory complications (P = 0.008). CONCLUSION: This study highlights similarities and differences in clinical and genetic spectrum of the most common PAD-associated gene defects. This comprehensive comparison will facilitate clinical decision making, and improve prognosis and targeted treatment

Clinical, immunologic and molecular spectrum of patients with immunodeficiency, centromeric instability, and facial anomalies (ICF) syndrome: A systematic review

Background: Immunodeficiency, centromeric instability and facial dysmorphism (ICF) syndrome is a rare autosomal recessive immune disorder presenting with hypogammaglobulinemia, developmental delay, and facial anomalies. The ICF type 1, type 2, type 3 and type 4 are characterized by mutations in DNMT3B, ZBTB24, CDCA7 or HELLS gene, respectively. This study aimed to present a comprehensive description of the clinical, immunologic and genetic features of patients with ICF syndrome. Methods: PubMed, Web of Science, and Scopus were searched systemically to find eligible studies. Results: Forty-eight studies with 118 ICF patients who met the inclusion criteria were included in our study. Among these patients, 60% reported with ICF-1, 30% with ICF-2, 4% with ICF-3, and 6% with ICF-4. The four most common symptoms reported in patients with ICF syndrome were: delay in motor development, low birth weight, chronic infections, and diarrhea. Intellectual disability and preterm birth among patients with ICF-2 and failure to thrive, sepsis and fungal infections among patients with ICF-1 were also more frequent. Moreover, the median levels of all three immunoglobulins (IgA, IgG, IgM) were markedly reduced within four types of ICF syndrome. Conclusion: The frequency of diagnosed patients with ICF syndrome has increased. Early diagnosis of ICF is important since immunoglobulin supplementation or allogeneic stem cell transplantation can improve the disease-free survival rate

Evaluation of the TLR negative regulatory network in CVID patients

Common variable immunodeficiency (CVID), a clinically symptomatic primary immunodeficiency disease (PID), is characterized by hypogammaglobulinemia leading to recurrent infections and various complications. Recently, some defects in the signaling of TLRs have been identified in CVID patients which led us to investigate the expression of TLR4 and 9 negative regulatory molecules and their upregulation status following their activation. Using TaqMan real-time PCR, SOCS1, TNFAIP3, RFN216, and IRAK-M transcripts among peripheral blood mononuclear cells (PBMCs) were measured with/without TLR4 and 9 activations. TLR4 and 9 were activated by lipopolysaccharide (LPS) and unmethylated CpG-oligodeoxynucleotide (CpG-ODN), respectively. Production of IFN-α and TNF-α cytokines, as a part of the functional response of mentioned TLRs, was also measured using ELISA. Deficient transcripts of IRAK-M and TNFAIP3 in unstimulated PBMCs and lower production of TNF-α and IFN-α after treatments were observed. Upregulation of RFN216 and TNFAIP3 after TLR9 activation was abnormal compared to healthy individuals. Significant correlations were found between abnormal IRAK-M and TNFAIP3 transcripts, and lymphadenopathy and inflammatory scenarios in patients, respectively. It seems that the transcriptional status of some negative regulatory molecules is disturbed in CVID patients, and this could be caused by the underlying pathogenesis of CVID and could involve complications like autoimmunity and inflammatory responses. © 2018, Macmillan Publishers Limited, part of Springer Nature

Evaluation of the TLR negative regulatory network in CVID patients

Common variable immunodeficiency (CVID), a clinically symptomatic primary immunodeficiency disease (PID), is characterized by hypogammaglobulinemia leading to recurrent infections and various complications. Recently, some defects in the signaling of TLRs have been identified in CVID patients which led us to investigate the expression of TLR4 and 9 negative regulatory molecules and their upregulation status following their activation. Using TaqMan real-time PCR, SOCS1, TNFAIP3, RFN216, and IRAK-M transcripts among peripheral blood mononuclear cells (PBMCs) were measured with/without TLR4 and 9 activations. TLR4 and 9 were activated by lipopolysaccharide (LPS) and unmethylated CpG-oligodeoxynucleotide (CpG-ODN), respectively. Production of IFN-α and TNF-α cytokines, as a part of the functional response of mentioned TLRs, was also measured using ELISA. Deficient transcripts of IRAK-M and TNFAIP3 in unstimulated PBMCs and lower production of TNF-α and IFN-α after treatments were observed. Upregulation of RFN216 and TNFAIP3 after TLR9 activation was abnormal compared to healthy individuals. Significant correlations were found between abnormal IRAK-M and TNFAIP3 transcripts, and lymphadenopathy and inflammatory scenarios in patients, respectively. It seems that the transcriptional status of some negative regulatory molecules is disturbed in CVID patients, and this could be caused by the underlying pathogenesis of CVID and could involve complications like autoimmunity and inflammatory responses. © 2018 Macmillan Publishers Limited, part of Springer Natur

Evaluation of the TLR negative regulatory network in CVID patients

Common variable immunodeficiency (CVID), a clinically symptomatic primary immunodeficiency disease (PID), is characterized by hypogammaglobulinemia leading to recurrent infections and various complications. Recently, some defects in the signaling of TLRs have been identified in CVID patients which led us to investigate the expression of TLR4 and 9 negative regulatory molecules and their upregulation status following their activation. Using TaqMan real-time PCR, SOCS1, TNFAIP3, RFN216, and IRAK-M transcripts among peripheral blood mononuclear cells (PBMCs) were measured with/without TLR4 and 9 activations. TLR4 and 9 were activated by lipopolysaccharide (LPS) and unmethylated CpG-oligodeoxynucleotide (CpG-ODN), respectively. Production of IFN-α and TNF-α cytokines, as a part of the functional response of mentioned TLRs, was also measured using ELISA. Deficient transcripts of IRAK-M and TNFAIP3 in unstimulated PBMCs and lower production of TNF-α and IFN-α after treatments were observed. Upregulation of RFN216 and TNFAIP3 after TLR9 activation was abnormal compared to healthy individuals. Significant correlations were found between abnormal IRAK-M and TNFAIP3 transcripts, and lymphadenopathy and inflammatory scenarios in patients, respectively. It seems that the transcriptional status of some negative regulatory molecules is disturbed in CVID patients, and this could be caused by the underlying pathogenesis of CVID and could involve complications like autoimmunity and inflammatory responses. © 2018 Macmillan Publishers Limited, part of Springer Natur

Comparison of clinical and immunological features and mortality in common variable immunodeficiency and agammaglobulinemia patients

Common Variable Immunodeficiency (CVID)and agammaglobulinemia are two of the main types of symptomatic primary antibody deficiencies. The pathogenic origins of these two diseases are different; agammaglobulinemia is a group of inherited disorders that usually are caused by mutations in the gene encoding Bruton Tyrosine Kinase (BTK)protein while CVID is a heterogeneous disorder mainly without monogenic cause. However, both diseases share a characteristic of frequent bacterial infections, a decline in serum immunoglobulin levels, and abnormality in antibody responses. The demographics and immunologic parameters, clinical manifestation, and mortality statistics from 297 patients with CVID and agammaglobulinemia followed up over 2 decades in the Children's Medical Center of Iran. Age at onset of symptom in agammaglobulinemia was earlier than CVID but the course of disease in CVID patients was longer than agammaglobulinemia patients. Pulmonary infections were the most prevalent clinical manifestations in both groups of patients. Lymphadenopathy, hepatomegaly, and splenomegaly were significantly higher in CVID patients than agammaglobulinemia patients and there was a significant association between these complications and mortality in CVID patients. Among 297 patients, 128 patients (88 CVID and 40 agammaglobulinemia)deceased. The predominant causes of death in CVID patients were infections, chronic lung disease, and malignancy while in agammaglobulinemia patients were infections and respiratory failure. Infections, especially respiratory infections were the most common complication and cause of death in both CVID and agammaglobulinemia groups and recent treatment advances even Immunoglobulin replacement cannot completely control these complications. Thus prompt recognition and specific management of these complications are worthwhile. © 2019 European Federation of Immunological Societie

The clinical and immunological features of patients with primary antibody deficiencies

Background: Primary antibody deficiency (PAD) comprises a range of diseases from early to late terminal B cells defects and is associated with the various clinical complications. Methods: A total of 461 patients (311 males and 150 females) with PADs enrolled in the retrospective cohort study and for all patients� demographic information, clinical records and laboratory data were collected to investigate clinical complications. Results: The most prevalent first presentations of immunodeficiency were respiratory tract infections in 63.5 and chronic diarrhea in 17.2. Common variable immune deficiency (CVID) patients had a higher diagnostic delay than class switching defect (CSD), and agammaglobulinemia. Among the non-infectious complications, autoimmunity (26.2), and splenomegaly (23.4) were the most common. Lymphadenopathy was higher in CSD patients than other PADs, while splenomegaly, hepatomegaly, autoimmunity and bronchiectasis were more common in CVID patients than others. Atopic manifestations were mostly recorded in patients with selective IgA deficiency. Malignancy was only reported in 5.8 of patients with CVID. There was a higher prevalence of autoimmune manifestations in CVID comparing to other PADs. Conclusion: PADs are relatively rare diseases and these patients have a variety of first clinical manifestations, such as diverse infections, autoimmunity, lymphoproliferation, allergy, enteropathy and malignancy. Practitioner�s awareness about the heterogeneous presentations of PAD disorders is poor, therefore patients often are lately diagnosed, and they are complicated with several clinical complications before the certain diagnosis. © 2018 Bentham Science Publishers

Evaluation of Radiation Sensitivity in Patients with Hyper IgM Syndrome

Background: HIGM syndrome is a rare form of primary immunodeficiencies characterized by normal/increased amounts of serum IgM and decreased serum levels of other switched immunoglobulin classes. Since the affected patients are continuously infected with various types of pathogens and are susceptible for cancers, diagnostic and therapeutic tests including imaging techniques are recommended for the diagnosis and treatment of these patients, which predispose them to higher accumulated doses of radiation. Given the evidence of class switching recombination machinery defect and its association with an increased rate of DNA repair, we aimed to evaluate radiation sensitivity among a group of patients diagnosed with HIGM syndrome. Methods: 19 HIGM patients (14 CD40 L and 3 AID deficiencies and 2 unsolved cases without known genetic defects) and 17 control subjects (10 healthy subjects as negative control group, 7 ataxia-telangiectasia patients as positive control group) were enrolled. G2 assay was carried out for the determination of radiosensitivity. Results: Based on radiation-induced chromosomal changes among the studied HIGM patients and their comparison with the controls, almost all (95) the patients had degrees of radiosensitivity: 6 patients with low to moderate, 1 patient with moderate, 11 patients with severe and 1 patient without radiation sensitivity. Conclusion: Today, X-ray radiation plays a very important role in diagnostic and therapeutic procedures; while increased exposure has devastating effects especially in radiosensitive patients. Considering higher sensitivity in HIGM patients, utilizing radiation-free techniques could partly avoid unnecessary and high-level exposure to radiation, thus preventing or reducing its harmful effects on the affected patients. © 2020, © 2020 Taylor & Francis Group, LLC